Fig. 1
Generation of HADHA Mutant and Knockout stem cell derived cardiomyocytes. a Schematic of fatty acid beta-oxidation detailing the four enzymatic steps. b Schematic of HADHA KO DNA and protein sequence from WTC iPSC line showing a 22 bp deletion, which resulted in an early stop codon. c Schematic of HADHA Mut DNA and protein sequence from WTC iPSC line showing a 2 bp deletion and 9 bp insertion on the first allele and a 2 bp deletion on the second allele. RNA-Sequencing read counts show that the HADHA Mut expresses exons 4–20 resulting in a truncated protein. d Western analysis of HADHA expression and housekeeping protein β-Actin in WTC iPSCs. e Confocal microscopy of WT, HADHA Mut and HADHA KO hiPSC-CMs for the cardiac marker αActinin (green) and HADHA (red). f Seahorse analysis trace of fatty acid oxidation capacity of WT, HADHA Mut and HADHA KO hiPSC-CMs. n = 6–7 biological replicates. Source data are provided as a Source Data file
